ABSTRACT
PURPOSE: This study assessed the long term survival rate and long term complications of patients who had a modified Fontan operation for functionally univentricular cardiac anomaly. METHODS: Between June 1986 and December 2000, 302 patients with a functional single ventricle underwent surgical interventions and were followed up until February 2006. The mean follow-up period was 8.3+/-5.3 years (range 3.5-18 years). Their median age was 2.4 years at the Fontan operation. The survival rate, the incidence and the risk factor of late complications were evaluated retrospectively. RESULTS: The verall survival rate was 91 percent at 5 years and 87 percent at 10 years. In multivariate analysis, early calendar year of operation and significant regurgitation were risk factors of death. The surviving patients showed NYHA functional class I in 82 percent, class II in 15 percent, and class III in 3 percent. Redo Fontan operations were necessary in 8.8 percent of patients at average 12.8+/-3.6 years after initial Fontan operation. The most common cause of Fontan conversion was atrial arrhythmia. The incidence of thromboembolic events was 9.3% and these complications were associated with the occurrence of atrial tachyarrhythmia. Supraventricular tachycardia including atrial flutter or fibrillation were reported on the follow-up examination by 11.2 percent of survivors after 8.4+/-5.6 years. Atriopulmonary connection showed higher rates of late tachycardia than lateral tunnel operation. CONCLUSIONS: This study revealed that the recent survival rate of Fontan type operation was satisfactory, but the occurrence of late complications after a Fontan type operation increased with the longer survival. There is a need for strict follow up and early treatment of late complications in patients who had a Fontan operation.